Hemoglobin S/OArab: Retinal Manifestations of a Rare Hemoglobinopathy

Abstract

Hemoglobin S/OArab (Hgb S/OArab) disease is a rare hemoglobinopathy which presents similarly to sickle cell retinopathy, with only three prior reports that describe associated retinal findings. In this report, we present ophthalmic examination findings in 2 patients with Hgb S/OArab. One patient exhibited peripheral ischemia and sunburst lesions without neovascular disease, and the other patient developed proliferative retinopathy of both eyes and multiple posterior-pole branch retinal artery occlusions in one eye. To our knowledge, this is the first case of retinal arterial occlusive disease in Hgb S/OArab, and the first report of fundus autofluorescence and OCT angiography in Hgb/OArab retinopathy.